Scleritis is a severe, destructive, vision-threatening inflammation involving the deep episclera and sclera. Symptoms are moderate to marked pain, hyperemia of the globe, lacrimation, and photophobia. Diagnosis is clinical. Treatment is with systemic corticosteroids and possibly immunosuppressants.

Scleritis is most common among women aged 30 to 50 yr, and many have connective tissue diseases, such as RA, SLE, polyarteritis nodosa, granulomatosis with polyangiitis (formerly called Wegener granulomatosis), or relapsing polychondritis. A few cases are infectious in origin. About half of the cases of scleritis have no known cause. Scleritis most commonly involves the anterior segment and occurs in 3 types—diffuse, nodular, and necrotizing.

Symptoms and Signs

Pain (often characterized as a deep, boring ache) is severe enough to interfere with sleep and appetite. Photophobia and lacrimation may occur. Hyperemic patches develop deep beneath the bulbar conjunctiva and are more violaceous than those of episcleritis or conjunctivitis. The palpebral conjunctiva is normal. The involved area may be focal (usually one quadrant of the globe) or involve the entire globe and may contain a hyperemic, edematous, raised nodule (nodular scleritis) or an avascular area (necrotizing scleritis). Posterior scleritis is less common and is less likely to cause red eye but more likely to cause blurred or decreased vision.

In severe cases of necrotizing scleritis, perforation of the globe and loss of the eye may result. Connective tissue disease occurs in 20% of patients with diffuse or nodular scleritis and in 50% of patients with necrotizing scleritis. Necrotizing scleritis in patients with connective tissue disease signals underlying systemic vasculitis.

Diagnosis

Clinical evaluation

Diagnosis of scleritis is made clinically and by slit-lamp examination. Smears or rarely biopsies are necessary to confirm infectious scleritis. CT or ultrasonography may be needed for posterior scleritis.

Prognosis

Of patients with scleritis, 14% lose significant visual acuity within 1 yr, and 30% lose significant visual acuity within 3 yr. Patients with necrotizing scleritis and underlying systemic vasculitis have a mortality rate of up to 50% in 10 yr (mostly due to MI).

Treatment

Systemic corticosteroids

Rarely, NSAIDs are sufficient for mild cases. However, usually a systemic corticosteroid (eg, prednisone 1 to 2 mg/kg po once/day for 7 days, then tapered off by day 10) is the initial therapy. If patients are unresponsive to or intolerant of systemic corticosteroids or have necrotizing scleritis and connective tissue disease, systemic immunosuppression with cyclophosphamide, methotrexate, or biologic agents (eg, rituximab) is indicated but only in consultation with a rheumatologist. Scleral grafts may be indicated for threatened perforation.

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